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Ophthalmologic manifestations of organoid nevus syndrome: A series of 13 cases.

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Senior Resident (Oculoplasty, Tumor & Pediatric Ophthalmology Services), Dr R. P. Centre for Ophthalmic Sciences, AIIMS, New Delhi, India.

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  • Organoid nevus syndrome is a rare neurocutaneous condition characterized by sebaceous nevus, seizures, and associated ocular abnormalities.
  • A study involving 13 patients showed that all had sebaceous nevus, with some also experiencing alopecia, seizures, and arachnoid cysts.
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  • Epibulbar choristoma is a benign congenital lesion with normal tissue but in the wrong location, categorized into three types based on histology: dermoid, dermolipoma, and complex choristoma.
  • In this case, a 9-year-old girl had a mass initially thought to be an epibulbar dermolipoma, which after surgery was revealed to be a complex choristoma upon histological examination.
  • The case highlights the need for orbital surgeons to recognize potential ossification in these lesions while providing clarity on the classification system for better diagnosis and management.*
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Rationale: A choristoma is a rare and benign neoplasm characterized by the presence of normal tissue in an anomalous anatomical location. In contrast, choristoma tend to occur in other body regions rather than within the spinal canal. Before our findings, only 4 cases of intraspinal choristoma had been recorded.

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